Iron Overload and Chelation Therapy

As early diagnosis and treatment of thalassemia are improving the prognosis of pediatric and young adult thalassemia patients, the major cause of illness and mortality has shifted from the problems of hemoglobin-deficient anemia to iron overload associated with chronic blood transfusion therapy. Heart, liver and other organ failure due to iron toxicity is the leading cause of death for thalassemia patients in the developed world. In chronically-transfused thalassemia patients, the body is receiving large amounts of exogenous iron and, having no mechanism to excrete the extra iron, stores it primarily in the spleen, liver, endocrine organs, and heart. Iron storage is further compounded by increased iron absorption from food as a way to combat anemia.

Aggressive monitoring of body iron burden is key to the survival and well-being of a chronically-transfused patient. There are several methods of iron assessment. The easiest, most affordable, least reliable and most common method is a serum ferritin test. This involves drawing a small sample of blood and testing for its ferritin content, which is an important iron-storage protein. Ferritin tests are useful for measuring gross iron overload or dramatic reduction in iron levels, but are limited when attempting to finely measure actual organ storage of iron. Several factors can affect test results: inflammation or infection of the liver, liver disease (such as fibrosis or hepatitis), hemolysis or breakdown of red blood cells during the blood draw or due to rough handling of the sample, vitamin C deficiency, or even too much alcohol the night before a test. Ferritin tests remain an approximation rather than an accurate indicator of iron stores. <next>

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To learn more about Iron Overload, including treatment recommendations and systems effected by iron-overload, please see pages 7-11 of our Standard of Care Guidelines.